methylmalonate semialdehyde + NAD+ + CoA + H2O => propionyl-CoA + HCO3- + NADH + H+
2M3OPROA + NAD+ + CoA-SH + H2O → PROP-CoA + NADH + H+ + HCO3-
- Reactome
- R-HSA-70893
- Compartment
- mitochondrial matrix
- Rhea
- RHEA:76628
Inputs
- 2M3OPROACHEBI:62413, C4H5O3, mitochondrial matrix
- NAD+CHEBI:57540, C21H26N7O14P2, mitochondrial matrix
- CoA-SHCHEBI:57287, C21H32N7O16P3S, mitochondrial matrix
- H2OCHEBI:15377, H2O, mitochondrial matrix
Outputs
- PROP-CoACHEBI:57392, C24H36N7O17P3S, mitochondrial matrix
- NADHCHEBI:57945, C21H27N7O14P2, mitochondrial matrix
- H+CHEBI:15378, H, mitochondrial matrix
- HCO3-CHEBI:17544, CHO3, mitochondrial matrix
Catalysed by
- methylmalonate-semialdehyde dehydrogenase (acylating, NAD) activity of ALDH6A1 [mitochondrial matrix]EC 1.2.1.27; UniProt Q02252
Literature
- Molecular characterization of methylmalonate semialdehyde dehydrogenase deficiency J Inherit Metab Dis, 2000
- CoA-dependent methylmalonate-semialdehyde dehydrogenase, a unique member of the aldehyde dehydrogenase superfamily. cDNA cloning, evolutionary relationships, and tissue distribution J Biol Chem, 1992